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Von Willebrand disease

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https://www.readbyqxmd.com/read/28453889/discrepant-platelet-and-plasma-von-willebrand-factor-in-von-willebrand-disease-patients-with-p-pro2808leufs-24
#1
Mackenzie L Bowman, Fred G Pluthero, Angie Tuttle, Lara Casey, Ling Li, Hilary Christensen, K Sue Robinson, David Lillicrap, Walter H A Kahr, Paula James
BACKGROUND: A novel variant, p.Pro2808Leufs*24, in the von Willebrand factor (VWF) gene was previously identified in the Canadian von Willebrand disease (VWD) patient population. Clinical observations of type 3 VWD patients with this variant indicate a milder bleeding phenotype compared to other type 3 patients. OBJECTIVE: To assess the effect of the Pro2808Leufs*24 variant on the molecular pathogenesis of VWD and correlate with the phenotype observed in patients...
April 28, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28447419/controversies-in-the-diagnosis-of-type-1-von-willebrand-disease
#2
REVIEW
M L Bowman, P D James
von Willebrand disease (VWD) is the most common bleeding disorder. Type 1 VWD represents the majority of cases and results from a partial quantitative deficiency of von Willebrand factor (VWF). The diagnosis of Type 1 VWD presents many challenges, despite there being three established diagnostic criteria: a personal history of mucocutaneous bleeding, a family history and low VWF levels. These criteria do not always coexist, and there is great overlap of clinical phenotypes and laboratory parameters between healthy individuals and those with VWD...
May 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28446234/retinal-vessel-diameters-and-reactivity-in-diabetes-mellitus-and-or-cardiovascular-disease
#3
R Heitmar, G Y H Lip, R E Ryder, A D Blann
BACKGROUND: Retinal vessel calibre and vascular dilation/constriction in response to flicker light provocation may provide a measure distinguishing patients suffering from diabetes mellitus and/or cardiovascular disease. METHODS: One hundred and sixteen age and sex matched patients with diabetes mellitus (DM), cardiovascular disease (CVD) and both DM and CVD (DM + CVD) underwent systemic and intraocular pressure measurements. Retinal vessel calibres were assessed using a validated computer-based program to compute central retinal artery and vein equivalents (CRVE) from monochromatic retinal images...
April 26, 2017: Cardiovascular Diabetology
https://www.readbyqxmd.com/read/28441050/comparison-of-platelet-function-and-viscoelastic-test-results-between-healthy-dogs-and-dogs-with-naturally-occurring-chronic-kidney-disease
#4
Alicia Dudley, Julie K Byron, Mary Jo Burkhard, Emma Warry, Julien Guillaumin
OBJECTIVE To compare platelet function and viscoelastic test results between healthy dogs and dogs with chronic kidney disease (CKD) to assess whether dogs with CKD have platelet dysfunction and altered blood coagulation. ANIMALS 10 healthy control dogs and 11 dogs with naturally occurring CKD. PROCEDURES Blood and urine were collected once from each dog for a CBC, serum biochemical analysis, urinalysis, and determination of the urine protein-to-creatinine ratio, prothrombin time, activated partial thromboplastin time, plasma fibrinogen concentration, and antithrombin activity...
May 2017: American Journal of Veterinary Research
https://www.readbyqxmd.com/read/28436749/differential-diagnosis-of-neonatal-alloimmune-thrombocytopenia-type-2b-von-willebrand-disease
#5
Mathilde Penel-Page, Sandrine Meunier, Mathilde Fretigny, Sandra Le Quellec, Pierre Boisseau, Christine Vinciguerra, Catherine Ternisien, Lucia Rugeri
At birth, severe thrombocytopenia without context of infection should mainly suggest neonatal alloimmune thrombocytopenia (NAIT), especially in case of a platelet count below 20 GL(-1). We report two cases of severe neonatal thrombocytopenia, first suspected as being NAIT. Both had a platelet count below 20 GL(-1) with platelet clumps. The absence of alloantibodies and failure of platelet transfusion and intravenous immunoglobulins to improve the platelet count led to question the diagnosis and to evoke inherited bleeding disorders...
April 24, 2017: Platelets
https://www.readbyqxmd.com/read/28416507/thrombotic-thrombocytopenic-purpura
#6
Bérangère S Joly, Paul Coppo, Agnes Veyradier
Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic micro-angiopathy characterized by a microangiopathic hemolytic anemia, severe thrombocytopenia and organ ischemia linked to disseminated microvascular platelet rich-thrombi. TTP is specifically related to a severe deficiency in ADAMTS13 (A Disintegrin and Metalloprotease with ThromboSpondin type 1 repeats, member 13), the specific von Willebrand factor-cleaving pro-tease. ADAMTS13 deficiency is most frequently acquired via ADAMTS13 autoantibodies but rarely, it is inherited via mutations of ADAMTS13 gene...
April 17, 2017: Blood
https://www.readbyqxmd.com/read/28415932/the-effects-of-corrective-surgery-on-endothelial-biomarkers-and-anthropometric-data-in-children-with-congenital-heart-disease
#7
Hung-Tao Chung, Yu-Sheng Chang, Sui-Ling Liao, Shen-Hao Lai
Objective To investigate the influence of surgical correction on biomarkers of endothelial dysfunction in children with congenital heart disease and to evaluate anthropometric data. Methods Children with pulmonary hypertension (PH) or Tetralogy of Fallot (TOF) who were scheduled for corrective surgery were enrolled in this prospective study. Age-matched healthy children were included as controls. Demographic, haemodynamic and cardiac ultrasonography data were collected. Blood samples were taken pre-surgery, 24-48 hours post-surgery and again 3-6 months later...
April 2017: Journal of International Medical Research
https://www.readbyqxmd.com/read/28409234/clinical-role-of-von-willebrand-factor-in-acute-ischemic-stroke
#8
Marija Menih, Miljenko Križmarić, Tanja Hojs Fabjan
BACKGROUND: An elevated level of von Willebrand factor (VWF) is associated with an increased risk for coronary heart disease and ischemic stroke. The objective of the study was to determine whether the level of VWF is associated with the cardioembolic subtype of ischemic stroke, stroke severity, and clinical outcome. PATIENTS AND METHODS: In this study 108 patients suffering from acute ischemic stroke (AIS) were included. According to the etiology of the stroke, patients were classified into the subtype of cardioembolic (CE) stroke and the group with non-CE stroke...
April 13, 2017: Wiener Klinische Wochenschrift
https://www.readbyqxmd.com/read/28405326/von-willebrand-factor-and-alkaline-phosphatase-predict-re-transplantation-free-survival-after-the-first-liver-transplantation
#9
Andreas Wannhoff, Conrad Rauber, Kilian Friedrich, Christian Rupp, Wolfgang Stremmel, Karl Heinz Weiss, Peter Schemmer, Daniel N Gotthardt
BACKGROUND: After liver transplantation (LT), there are liver-related, infectious and cardiovascular complications that contribute to reduced graft survival. These conditions are associated with an increase in the Von Willebrand factor antigen (VWF-Ag), which was previously correlated with survival in cirrhotic patients. OBJECTIVE: Evaluate VWF-Ag as a predictive marker of re-transplantation-free survival in patients after LT. METHODS: We measured VWF-Ag in patients after first LT and then followed them prospectively with regard to the primary endpoint, namely re-transplantation-free survival...
February 2017: United European Gastroenterology Journal
https://www.readbyqxmd.com/read/28403644/the-effect-of-basal-insulin-glargine-on-the-fibrinolytic-system-and-von-willebrand-factor-in-people-with-dysglycaemia-and-high-risk-for-cardiovascular-events-swedish-substudy-of-the-outcome-reduction-with-an-initial-glargine-intervention-trial
#10
Aslak Rautio, Kurt Boman, Hertzel C Gerstein, Jenny Hernestål-Boman, Shun Fu Lee, Mona Olofsson, Linda Garcia Mellbin
INTRODUCTION: Fibrinolytic factors, plasminogen activator inhibitor-1, tissue plasminogen activator, tissue plasminogen activator/plasminogen activator-complex and the haemostatic factor von Willebrand factor are known markers of cardiovascular disease. Their plasma levels are adversely affected in patients with dysglycaemia, and glucose normalization with insulin glargine might improve the levels of these factors. METHODS: Prespecified Swedish substudy of the Outcome Reduction with an Initial Glargine Intervention trial (ClinicalTrials...
April 1, 2017: Diabetes & Vascular Disease Research
https://www.readbyqxmd.com/read/28395735/induced-pluripotent-stem-cells-derived-from-bernard-soulier-syndrome-patient-s-peripheral-blood-cells-with-a-p-phe55ser-mutation-in-the-gpix-gene
#11
Lourdes Lopez-Onieva, Mar Lamolda, Rosa Montes, Maria Luisa Lozano, Vicente Vicente, José Rivera, Verónica Ramos-Mejía, Pedro J Real
Bernard Soulier Syndrome (BSS) is a rare autosomal platelet disorder characterized by mutations in the von Willebrand factor platelet receptor complex GPIb-V-IX. In this work we have generated an induced pluripotent stem cell (BSS3-PBMC-iPS4F8) from peripheral blood mononuclear cells of a BSS patient with a p.Phe55Ser mutation in the GPIX gene. Characterization of BSS3-PBMC-iPS4F8 showed that these cells maintained the original mutation present in the BSS patient, expressed pluripotent stem cell markers and were able to differentiate into the three germline layers...
April 2017: Stem Cell Research
https://www.readbyqxmd.com/read/28394285/diagnosis-and-treatment-of-von-willebrand-disease-and-rare-bleeding-disorders
#12
REVIEW
Giancarlo Castaman, Silvia Linari
Along with haemophilia A and B, von Willebrand disease (VWD) and rare bleeding disorders (RBDs) cover all inherited bleeding disorders of coagulation. Bleeding tendency, which can range from extremely severe to mild, is the common symptom. VWD, due to a deficiency and/or abnormality of von Willebrand factor (VWF), represents the most frequent bleeding disorder, mostly inherited as an autosomal dominant trait. The diagnosis may be difficult, based on a bleeding history and different diagnostic assays, which evaluate the pleiotropic functions of VWF...
April 10, 2017: Journal of Clinical Medicine
https://www.readbyqxmd.com/read/28392945/pitfalls-in-interventional-pain-medicine-hyponatremia-after-ddavp-for-a-patient-with-von-willebrand-disease-undergoing-an-epidural-steroid-injection
#13
Talal W Khan, Abdulraheem Yacoub
Desmopressin (DDAVP), a synthetic analog of vasopressin, has been used in patients with von Willebrand disease (VWD), mild hemophilia A, and platelet dysfunction to reduce the risk of bleeding associated with surgical and interventional procedures. We report the case of a patient with VWD presenting with a bulging disc and radicular pain that underwent transforaminal epidural steroid injections. Her course was complicated with the interval development of headaches and dizziness symptomatic of moderate hyponatremia, likely due to excessive fluid intake...
2017: Case Reports in Anesthesiology
https://www.readbyqxmd.com/read/28388959/autosomal-recessive-inherited-bleeding-disorders-in-pakistan-a-cross-sectional-study-from-selected-regions
#14
Arshi Naz, Muhammad Younus Jamal, Samina Amanat, Ikram Din Ujjan, Akber Najmuddin, Humayun Patel, Fazle Raziq, Nisar Ahmed, Ayisha Imran, Tahir Sultan Shamsi
BACKGROUND: Autosomal recessive bleeding disorders (ARBDs) include deficiencies of clotting factors I, II, V, VII, X, XI, XIII, vitamin K dependent clotting factors, combined factor V & VIII, Von Willebrand Disease (vWD) type 3, Glanzmann's thrombasthenia (GT) and Bernard-Soulier syndrome. Patients with primary bleeding disorders from all the major provincial capitals of Pakistan were screened for ARBDs. Prothrombin (PT), activated partial thromboplastin time (APTT), bleeding time (BT) and fibrinogen levels were measured...
April 7, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28388906/predictors-of-quality-of-life-among-adolescents-and-young-adults-with-a-bleeding-disorder
#15
John M McLaughlin, James E Munn, Terry L Anderson, Angela Lambing, Bartholomew Tortella, Michelle L Witkop
BACKGROUND: Health-related quality of life (HRQoL) in adolescents and young adults with bleeding disorders is under-researched. We aimed to describe factors related to HRQoL in adolescents and young adults with hemophilia A or B or von Willebrand disease. METHODS: A convenience sample of volunteers aged 13 to 25 years with hemophilia or von Willebrand disease completed a cross-sectional survey that assessed Physical (PCS) and Mental (MCS) Component Summary scores on the SF-36 questionnaire...
April 7, 2017: Health and Quality of Life Outcomes
https://www.readbyqxmd.com/read/28382967/thrombotic-thrombocytopenic-purpura
#16
REVIEW
Johanna A Kremer Hovinga, Paul Coppo, Bernhard Lämmle, Joel L Moake, Toshiyuki Miyata, Karen Vanhoorelbeke
Thrombotic thrombocytopenic purpura (TTP; also known as Moschcowitz disease) is characterized by the concomitant occurrence of often severe thrombocytopenia, microangiopathic haemolytic anaemia and a variable degree of ischaemic organ damage, particularly affecting the brain, heart and kidneys. Acute TTP was almost universally fatal until the introduction of plasma therapy, which improved survival from <10% to 80-90%. However, patients who survive an acute episode are at high risk of relapse and of long-term morbidity...
April 6, 2017: Nature Reviews. Disease Primers
https://www.readbyqxmd.com/read/28379876/recombinant-von-willebrand-factor-for-severe-gastrointestinal-bleeding-unresponsive-to-other-treatments-in-a-patient-with-type-2a-von-willebrand-disease-a-case-report
#17
Racquel Brown
: A recombinant von Willebrand factor (rVWF) was recently approved in the United States for on-demand treatment and control of bleeding episodes in adults with von Willebrand disease (VWD). In contrast to plasma-derived VWF products available in the United States, rVWF does not contain factor VIII (FVIII). To date, there is no published experience of rVWF in clinical practice. We report the acute and prophylactic use of rVWF in a patient with VWD type 2A and severe gastrointestinal bleeding. Dosing with plasma-derived VWF/FVIII concentrates was constrained by FVIII accumulation; the bleeding was unresponsive, and multiple red blood cell transfusions were required...
April 4, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28374895/increased-von-willebrand-factor-over-decreased-adamts-13-activity-is-associated-with-poor-prognosis-in-patients-with-advanced-non-small-cell-lung-cancer
#18
Renyong Guo, Jiezuan Yang, Xia Liu, Jianping Wu, Yu Chen
BACKGROUND: Hypercoagulability induced by the imbalance between von Willebrand factor (VWF) secretion and its cleaving protease (ADAMTS-13) has been correlated with cancer metastasis. The aim of this study was to explore the prognostic significance of the VWF/ADAMTS-13 ratio in advanced non-small-cell lung cancer (NSCLC). METHODS: Pre-treatment sera/plasma levels of VWF, ADAMTS-13, VWF/ADAMTS-13 ratio, factor (F) VIII, and other clinical/laboratory parameters were measured in 119 patients with advanced NSCLC and 102 healthy controls...
April 4, 2017: Journal of Clinical Laboratory Analysis
https://www.readbyqxmd.com/read/28366840/role-of-altered-coagulation-fibrinolytic-system-in-the-pathophysiology-of-diabetic-retinopathy
#19
REVIEW
Tapan Behl, Thirumurthy Velpandian, Anita Kotwani
The implications of altered coagulation-fibrinolytic system in the pathophysiology of several vascular disorders, such as stroke and myocardial infarction, have been well researched upon and established. However, its role in the progression of diabetic retinopathy has not been explored much. Since a decade, it is known that hyperglycemia is associated with a hypercoagulated state and the various impairments it causes are well acknowledged as independent risk factors for the development of cardiovascular diseases...
March 30, 2017: Vascular Pharmacology
https://www.readbyqxmd.com/read/28362648/abnormal-angiogenesis-in-blood-outgrowth-endothelial-cells-derived-from-von-willebrand-disease-patients
#20
Soundarya N Selvam, Lara J Casey, Mackenzie L Bowman, Lindsey G Hawke, Avery J Longmore, Jeffrey Mewburn, Mark L Ormiston, Stephen L Archer, Donald H Maurice, Paula James
Bleeding associated with angiodysplasia is a common, often intractable complication in patients with von Willebrand disease (VWD). von Willebrand factor (VWF), the protein deficient or defective in VWD, is a negative regulator of angiogenesis, which may explain the pathologic blood vessel growth in VWD. This study explores the normal range of angiogenesis in blood outgrowth endothelial cells (BOECs) derived from healthy donors and compares this to angiogenesis in BOECs from VWD patients of all types and subtypes...
March 30, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
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